Classification of myasthenia gravis based on autoantibody status
Classification of myasthenia gravis based on autoantibody status
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DOI：
10.1001/archneur.64.8.1121
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发表时间：
2007-08-01
影响因子：
--
通讯作者：
Kuwana, Masataka
中科院分区：
文献类型：
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作者：
Suzuki, Shigeaki;Utsugisawa, Kimiaki;Kuwana, Masataka
目的：探讨重症肌无力（MG）患者的自身抗体状态，并评估其有用性疾病classification.Design：回顾性队列研究MG患者，谁有自身抗体受体和离子通道表达在神经肌肉接头和肌肉，损害神经肌肉传递。研究的自身抗体之一是最近发现的一种新的MG特异性自身抗体，其针对电压门控钾（Kv）通道Kv1.4。研究地点：东京庆应义塾大学医院和盛冈岩手医科大学医院。患者：209名MG患者。主要结果测量：用35硫标记的横纹肌肉瘤细胞提取物通过免疫沉淀法测定抗Kv1.4抗体。结果：209例MG患者中，抗乙酰胆碱受体抗体阳性150例（72%），抗Kv1.4抗体阳性26例（12%），抗titin抗体阳性50例（24%）。所有抗Kv1.4或抗titin抗体阳性的患者均为抗乙酰胆碱受体抗体阳性。根据患者的3种MG相关自身抗体（抗Kv1.4、抗肌联蛋白和抗乙酰胆碱受体）的状态将其分为4组。抗Kv1.4抗体与延髓受累、肌无力危象、胸腺瘤和伴随的心肌炎和/或肌炎之间存在临床相关性;抗肌联素抗体与老年型MG之间存在临床相关性;单独抗乙酰胆碱受体抗体与年轻型MG之间存在临床相关性;血清阴性与眼部MG之间存在临床相关性。此外，抗Kv 1.4组MG患者的疾病表现比其他3组更严重。结论：根据自身抗体状态对MG患者进行分类可能有助于定义临床子集。
Objectives: To investigate the autoantibody status of patients with myasthenia gravis (MG) and to evaluate its usefulness for disease classification.Design: Retrospective cohort study of patients with MG, who have autoantibodies to receptors and ion channels expressed at neuromuscular junctions and in muscles that impair neuromuscular transmission. One of the autoantibodies studied was a recently identified, novel, MG-specific autoantibody to a voltage-gated potassium (Kv) channel, Kv1.4.Setting: Keio University Hospital, Tokyo, and Iwate Medical University Hospital, Morioka.Patients: Two hundred nine patients with MG.Main Outcome Measures: Anti-Kv1.4 antibody was measured by an immunoprecipitation assay with sulfur 35-labeled extract from rhabdomyosarcoma cells. Antititin antibody was detected with a commercially available enzyme-linked immunosorbent assay.Results: Anti-acetylcholine receptor, anti-Kv1.4, and antititin antibodies were detected in 150 (72%), 26 (12%), and 50 (24%) of the 209 patients with MG, respectively. All of the patients who were positive for anti-Kv1.4 or antititin antibody were seropositive for the anti acetylcholine receptor antibody. They were classified into 4 groups based on their status in regard to 3 MG-related autoantibodies: anti-Kv1.4, antititin, and anti acetylcholine receptor. Clinical associations were found between anti-Kv1.4 and bulbar involvement, myasthenic crisis, thymoma, and concomitant myocarditis and/or myositis; between antititin and older-onset MG; between anti acetylcholine receptor alone and younger-onset MG; and between seronegativity and ocular MG. In addition, patients with MG in the anti-Kv1.4 group had more severe manifestations of disease than those in the other 3 groups.Conclusion: Classification of patients with MG based on autoantibody status may be useful in defining clinical subsets.